PRESENTING AS COARCTATION OF AORTA
AND RENOVASCULAR HYPERTENSION
WITH DISSECTION OF AORTA.
DR. ABHAY MANGE
A 16 yrs old female patient was admitted with c/o
Breathlessness since 2 months
Swelling over lower limbs since 15
Decreased urine output since 8
h/o fever since 1 month +
No h/o cough/chest
No h/o joint pain
No h/o intermittent claudication
No h/o DM/PTB /RHD in past
Conscious and oriented
Pulse -104/min in rt radial,regular,bounding
lt radial weak ,no r-r delay. femorals and popliteals
dorsalis pedis palpable, no radio-femoral delay.
Blood pressure(mmHg) :- RT. UL =210/110 LT. UL
RT. And LT. LL =240/130
Edema feet +
CVS:- PSM + III/VI AT APEX
RS:-Basal crepitations present
PA :-liver palpable 4cm /tender
Abdominal bruit +
CNS :- NAD
PS:-RBC‟S were normocytic normochromic with mild
anisopoikilocytosis,platelets –adequate,no parasite
ESR =36 mm in 1st hr.
URINE :Protein ++,sugar-nil,5-6 pus cells ,10-12 rbcs
Na =122 ,K=3.8 meq/l
LFT:-TP =6.2, TB=2.7,ALP=325,OT =66,PT=268
ANA :-0.29 OD RATIO
AORTOARTERITIS WITH RENAL ARTERY STENOSIS
WITH RENOVASCULAR HYPERTENSION WITH
SEVERE LV DYSFUNCTION.
MR Renal angiography
Study revels Type B standford aortic dissection at
level of origin of renal arteries ,2 cm in diameter and 2.1
cm in length
Lt. renal artery arising from true lumen
Rt. Renal artery arising from false lumen with significant
reduction in caliber.
Lt. kidney measure 9.6/4.7/5.2 cms and artery measure
6mm at ostia,3mm at hilum.
Rt . Kidney measure 7.2/3.6/3.3cms and artery measure
2.5mm at ostia,1.8mm at hilum
Takayasu‟s arteritis (TA) is an inflammatory vascular
disease of the young involving the large elastic arteries
resulting in occlusive or ecstatic changes mainly in the
aorta and its major branches as well as the pulmonary
artery and its branches.
Also known by “pulseless disease”, “occlusive
thromboaortopathy”, and “Martorell syndrome”,
predilection for females with wide geographical
M : F ratio in 1 : 6.4 in india
TA may be diagnosed from childhood to late adult life,
Rarely occurs over 40 years of age
1827 by R. Adams who was the first physician to note
absence of pulse in all four extremities
In1856 Savory reported a case of young female with
absence of pulses.
In 1908, Mikito Takayasu, a Japanese opthalmologist
described a peculiar wreath-like appearance of retinal
blood vessels with absence of radial pulse.
All the three layers causing extensive intimal
proliferation, inflammation of media and adventitia
followed by marked fibrous scarring.
In older cases, hyalinization of deeper layers of intima
and dystrophic calcification can be seen.
Skipped areas of aortic involvement are quite
characteristic of aortoarteritis.
Histopathologically, adventitial mononuclear infiltrate
with perivascular cuffing of the vasa vasorum,
mononuclear inflammation, and rarely granulomatous
The subclavian artery, common carotid artery, vertebral
artery, and renal artery are more often affected.
Early phase or pre pulseless phase described by
nonspecific systemic features (malaise, arthralgia mild
synovitis, weakness, myalgias weight loss, and low-grade
Pulseless phase (vascular inflammatory phase) is
characterized by claudication, headaches, dizziness, and
amaurosis or diplopia, difficulty in looking upwards,
renovascular hypertension, chest pain or palpitation,
pulmonary (dyspneea,hemoptysis and pleurisy),
gastrointestinal (anorexia, nausea), and skin (rare, ulcers).
Occlusive phase: usually involves common carotid artery
(visual defects, strokes, TIA), vertebral artery (dizziness,
visual defects), subclavian artery (arm claudication), aorta
(aortic regurgitation,cardiac heart failure), pulmonary artery,
cardiac and celiac axis, renal artery (renovascular
hypertension), iliac artery (claudication).
The diagnosis of TA is largely based on the combination
1) clinical manifestations,
2) laboratory evaluation, and
When the diagnosis is presumed, it is typically confirmed
by a radiographic technique.
American College OF Rheumatology
Criteria to be diagnosed with Takayasu’s
must meet 3 out of 6 criteria:
Age under 40 at disease onset;
Claudication of extremities;
Decreased branchial artery pulse;
Blood pressure difference>10 mmHg between arms;
Bruit over subclavian arteries or aorta;
Arteriogram abnormality: occlusion or narrowing in aorta
or main branches.
Diagnostic criteria by Ishikawa
These consist of one
obligatory criterion (age <40 years at diagnosis or onset of
two major criteria (left and right mid subclavian artery
nine minor criteria
(high ESR, common carotid artery tenderness, hypertension,
aortic regurgitation or annulo-aortic ecatisa, lesions of
pulmonary artery, left mid common carotid artery, distal
branchio-cephalic trunk, thoracic aorta and abdominal aorta).
In addition to the
obligatory criterion, the presence of two major criteria , or
one major plus two or more minor criteria; or four or
more minor criteria suggests a high probability of the
presence of aortoarteritis.
The Ishikawa diagnostic criteria modified
by Sharma et al 1995
Type I -only the branches of the aortic arch.n
Type IIa - aorta only at its ascendingportion and/or at the aortic arch. The
branches ofthe aortic arch may be involved as well. The rest ofthe aorta is
Type IIb - the descending thoracic aorta withor without involvement of the
ascending aorta orthe aortic arch with its branches. The abdominalaorta is
Type III is concomitent involvement of thedescending thoracic aorta, the
abdominal aorta,and/or the renal arteries. The ascending aorta, theaortic
arch, and its branches are not involved.
Type IV involves only the abdominal aorta and/orthe renal arteries.
Type V is a generalized type, with combined
Sharma modified criteria for clinical diagnosis
1. Left midsubclavian artery lesion: stenosis or occlusion 1 cm proximal to the left
vertebral artery orifice up to 3 cm distal
2. Right midsubclavian artery lesion: stenosis or occlusion from the right vertebral
artery orifice to 3 cm beyond
3. Characteristic signs and symptoms (> 1-month duration)
A. Limb claudication
B. Pulselessness or blood pressure differential > 10 mmHg in arms
C. Exercise ischaemia
D. Neck pain
F. Amaurosis fugax
J. Blurred vision
1. High ESR: Westergren ESR > 20 mm/h
3. Hypertension: brachial blood pressure > 140/90 mmHg o popliteal blood
pressure > 160/90 mmHg
4. Aortic regurgitation or annuloaortic ectasia: determined by auscultation,
arteriography or echocardiography
5. Pulmonary artery lesion: lobar or segmental artery occlusion, or stenosis or
aneurysm of pulmonary trunk
6. Left middle common carotid artery lesion: stenosis or occlusion of middle 5
cm portion starting 2 cm from its orifice
7. Distal innominate artery lesion: stenosis or occlusion in the distal third
8. Descending thoracic aorta lesion: narrowing, aneurysm, or luminal
9. Abdominal aortic lesion: narrowing, aneurysm, or luminal irregularity
10. Coronary artery lesion: documented by arteriography in patients < 30 years
of age and without risk factors for atherosclerosis
Two major, or one major and two minor, or four minor
criteria indicate a high probability of Takayasu‟s arteritis.
Clinical Classification 2004
Ueno et 1967 classified it into three types.
Type-I, localized to the arch of aorta and its branches.
Type-II,involve the thoracoabdominal aorta and its branches
without involvement of the arch.
Type-III is combined features of both Type-I and Type-II.
Type-IV in which there is pulmonary involvement in addition to
features of Type-I, II or III.( Lupi Harrera et al in 1975)
Type- V proposed by Panja et al (2004) and it indicates
involvement of coronary arteries.
The distribution of various types is not uniform globally.
As far as India is concerned, Agarwal et al reported among North
Indian patients the
Incidence of various types as
Type-I - 22%,
Type-II - 25%,
Type-III - 53% and
Type-IV - 26%.
Panja et al involving 650 patients belonging to Eastern India and
Bangladesh (largest series from India) revealed
Type- I - 16%;
Type-II - 8%;
Type-III - 76%;
Type-IV - 36% and
Type-V - 10%.
Angiography is investigation of choice for the diagnostic
evaluation of TA
Echocardiography and Duplex Doppler
ultrasonography are the first investigations and the
characteristic findings of TA consist of wall
thickening,stenosis, aneurysms, calcifications,
occlusions, and pulsatility.
Computed tomography (CT) and magnetic resonance
imaging (MRI) are the best diagnostic techniques for
assessing pulmonary vascular lesions
PET technique or FDG-PET ((18) F-fluorodeoxyglucose
positron emission tomography permits an early diagnosis
of TA during the inflammatory or „„pre-pulseless‟‟ phase
High-dose corticosteroids are the mainstay of TA
therapy if caught early ; consists of prednisone 1
mg/kg/day given for the first 1–3 months, then reduction
after an alternate-day
Cytotoxic therapy is mainly used in cases with ongoing
disease activity, regardless of glucocorticoid therapy.
cyclophosphamide (1-2 mg/kg/day), azathioprine (1-2
mg/kg/day), or methotrexate (0.3 mg/kg/week)
Recently, mycophenolate mofetil, and anti-tumour
necrosis factor alpha biological agents have been
proposed in patients unresponsive to other
Indications for surgical repair or angioplasty are as
1.Renovascular stenosis causing hypertension
2.Coronary artery stenosis leading to myocardial ischemia
3.Extremity claudication induced by routine activity
4.Cerebral ischemia and/or critical stenosis of 3 or more
6.Thoracic or abdominal aneurysms larger than 5 cm in
7.Severe coarctation of the aorta
8.Percutaneous transluminal coronary angioplasty is followed
by restenosis at the angioplasty site within 1-2 years in a
substantial number of patients.
TAKAYASU’S ARTERITIS – AN UPDATE- Daniela
Bartoº1,Ecaterina Bontaº1,S. Ghiorghe1 Cercetãri
Experimentale & Medico-Chirurgicale Anul XIII l Nr3-
4/2006 l Pag. 149-152
Takayasu’s arteritis: a review of the literature -Silvia
Maffei, Michela Di Renzo, (Intern Emerg Med 2006; 1
Current Status of Aortoarteritis in India Manotosh
Panja*, PC Mondal**
Aortoarteritis presenting as coarctation of aorta.-
Bahuleyan CG, Bhuvaneswaran KP